颞骨朗格汉斯组织细胞增生症临床分析

Clinical Features of Langerhans Cell Histiocytosis of Temporal Bone

目的分析颞骨朗格汉斯组织细胞增生症（Langerhans cell histiocytosis,LCH）的临床特点、诊断、治疗和预后。方法回顾性收集解放军总医院耳鼻咽喉头颈外科2009年6月至2014年6月收治并经病理证实的颞骨LCH作为研究对象,按2009年国际组织细胞协会提出的诊断标准、临床分类和疗效评定标准,分析其临床表现、诊断、治疗及预后等临床特点,根据年龄、临床分类分组,比较组间预后差异。结果本组颞骨LCH主要表现为耳漏、耳聋、耳颞部包块、肉芽肿等;16例患者中,幼儿组10例（年龄≤4岁）,成人组6例（年龄24岁～68岁）,经统计分析组间预后差异无统计学意义。按国际组织细胞协会的分类,多系统组4例,单系统组12例,单系统组预后优于多系统组,差异有统计学意义。结论颞骨LCH临床表现多样,以耳漏和耳聋为常见症状而面神经极少受累,确诊主要靠病理。手术切除是首选治疗方法,颞骨LCH单系统受累患者预后优于多系统受累者。

Objective Temporal bone Langerhans Cell Histiocytosis （LCH） is mainly characterized by otorrhea, mas- toid mass, granuloma in the middle ear and external auditory canal, and hearing loss, while facial paralysis is rare. This study is aimed at analyzing clinical and prognostic features of temporal bone LCH. Methods Clinical data from 16 cases of LCH of the temporal bone treated from July 2009 to July 2014 at the Chinese PLA General Hospital were reviewed, including fol- lowing ups clinical presentation, ,The 2009 International Histocyte Society guidelines for diagnosis, clinical classification and outcome evaluation were adopted in the analysis. Correlation between age and clinical classifications and prognosis was also analyzed. Results Among the 16 patients, 10 were children （≤ 4 years old） and 6 were adults （24 - 68 years old）. Multiple system involvement was seen in 4 of the 16 patients, and the rest patients showed only single system involvement. While age was not correlated to treatment outcomes, clinical classification appeared to be correlated to the prognosis. Conclusion The number of organ affected by LCH may be a good prognosis indicator in these patients.